ATP-dependent RNA helicase SUPV3L1, mitochondrial

Definition:

Major helicase player in mitochondrial RNA metabolism. Component of the mitochondrial degradosome (mtEXO) complex, that degrades 3' overhang double-stranded RNA with a 3'-to-5' directionality in an ATP-dependent manner. Involved in the degradation of non-coding mitochondrial transcripts (MT-ncRNA) and tRNA-like molecules. ATPase and ATP-dependent multisubstrate helicase, able to unwind double-stranded (ds) DNA and RNA, and RNA/DNA heteroduplexes in the 5'-to-3' direction. Plays a role in the RNA surveillance system in mitochondria; regulates the stability of mature mRNAs, the removal of aberrantly formed mRNAs and the rapid degradation of non coding processing intermediates. Also implicated in recombination and chromatin maintenance pathways. May protect cells from apoptosis. Associates with mitochondrial DNA.

References:

[1]. Lily Khidr, et al. Role of SUV3 helicase in maintaining mitochondrial homeostasis in human cells. J Biol Chem. 2008 Oct 3;283(40):27064-73. [Content Brief]

[2]. Zhanyong Shu, et al. Purified human SUV3p exhibits multiple-substrate unwinding activity upon conformational change. Biochemistry. 2004 Apr 27;43(16):4781-90. [Content Brief]

[3]. Zbigniew Pietras, et al. Dedicated surveillance mechanism controls G-quadruplex forming non-coding RNAs in human mitochondria. Nat Commun. 2018 Jul 2;9(1):2558. [Content Brief]

[4]. Roman J Szczesny, et al. Down-regulation of human RNA/DNA helicase SUV3 induces apoptosis by a caspase- and AIF-dependent pathway. Biol Cell. 2007 Jun;99(6):323-32. [Content Brief]

[5]. Roman J Szczesny, et al. Human mitochondrial RNA turnover caught in flagranti: involvement of hSuv3p helicase in RNA surveillance. Nucleic Acids Res. 2010 Jan;38(1):279-98. [Content Brief]

[6]. Michal Minczuk, et al. Localisation of the human hSuv3p helicase in the mitochondrial matrix and its preferential unwinding of dsDNA. Nucleic Acids Res. 2002 Dec 1;30(23):5074-86. [Content Brief]

[7]. Mandy Pereira, et al. Interaction of human SUV3 RNA/DNA helicase with BLM helicase; loss of the SUV3 gene results in mouse embryonic lethality. Mech Ageing Dev. 2007 Nov-Dec;128(11-12):609-17. [Content Brief]

[8]. Dennis Ding-Hwa Wang, et al. Human mitochondrial SUV3 and polynucleotide phosphorylase form a 330-kDa heteropentamer to cooperatively degrade double-stranded RNA with a 3'-to-5' directionality. J Biol Chem. 2009 Jul 31;284(31):20812-21. [Content Brief]

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